Prognostic relevance of anemia and transfusion dependency in myelodysplastic syndromes and primary myelofibrosis.

نویسندگان

  • Animesh Pardanani
  • Ayalew Tefferi
چکیده

Malaventura C, et al. Deferasirox, deferiprone and desferrioxamine treatment in thalassemia major patients: cardiac iron and function comparison determined by quantitative magnetic resonance imaging. Comparison of effects of oral deferiprone and subcutaneous desfer-rioxamine on myocardial iron concentrations and ventricular function in beta-thalassaemia. Effects of chelation therapy on cardiac function improvement in thalassemia patients: literature review and the Taiwanese experience. Comparative effects of deferiprone and deferoxamine on survival and cardiac disease in patients with thalassemia major: a retrospective analysis. et al. Randomized controlled trial of deferiprone or deferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis. et al. Cardiac morbidity and mortality in deferoxamine-or deferiprone-treated patients with thalassemia major. Berdoussi E, et al. Relation of chelation regimes to cardiac mortality and morbidity in patients with thalassaemia major: an observational study from a large Greek Unit. Eur J Haematol.85(4):335-44. The efficacy of iron chelator regimes in reducing cardiac and hepatic iron in patients with thalassaemia major: a clinical observational study. al. Improving survival with deferiprone treatment in patients with thalassemia major: a prospective multicenter randomised clinical trial under the auspices of the Italian Society for Thalassemia and Hemoglobinopathies. et al. The effect of deferasirox on cardiac iron in thalassemia major: impact of total body iron stores. Aydinok Y, et al. Continued improvement in myocardial T2* over 2 years of deferasirox therapy in {beta}-thalassemia major patients with cardiac iron overload. Harmatz P, et al. Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel. Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalas-saemia major. A nemia is a cardinal manifestation of myelodysplas-tic syndromes (MDS) and primary myelofibrosis (PMF). Over half (54%) of the patients with de novo untreated myelodysplastic syndromes, in the International Prognostic Scoring System (IPSS) cohort, presented with hemoglobin levels of less than 10 g/dL. 1 In another independent cohort with IPSS low or intermediate -1 risk myelodysplastic syndromes, an almost identical proportion (55%) of patients presented with a similar degree of anemia. 2 Similarly, most patients with primary myelofibrosis are anemic at presentation and the hemo-globin level was less than 10 g/dL in 35% to 54% of patients in some studies. The pathogenesis of anemia in both myelodysplastic syndromes and primary myelofibrosis is poorly understood and is attributed to " ineffective erythropoiesis " for convenience. Because both disorders are markedly heterogeneous in their molecular and biological features, …

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Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade®, ICL670)

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عنوان ژورنال:
  • Haematologica

دوره 96 1  شماره 

صفحات  -

تاریخ انتشار 2011